Jachiet Vincent, Laine Laurent, Gendre Thierry, Henry Carole, Da Silva Daniel, de Montmollin Etienne
Intensive Care Unit, Centre Hospitalier de Saint-Denis, 2 Rue du Docteur Delafontaine, 93200, Saint Denis, France.
Neurology Department, Centre Hospitalier de Saint-Denis, Saint Denis, France.
Neurocrit Care. 2016 Dec;25(3):455-457. doi: 10.1007/s12028-016-0296-0.
Stiff-person syndrome (SPS) is a rare disorder characterized by progressive muscle stiffness, rigidity, and spasms involving the axial muscles. Acute respiratory distress has rarely been reported in this condition.
We report a case of a 49-year-old woman with autoimmune SPS diagnosed during an episode of acute respiratory failure secondary to repetitive episodes of apnea, requiring intensive care.
Acute respiratory failure manifesting with apneic episodes is a life-threatening and unpredictable complication of SPS. Its pathophysiology is not well known. The two suggested mechanisms are as follows: (1) apnea due to muscle rigidity and paroxysmal muscle spasms, and (2) paroxysmal autonomic hyperactivity. Sudden and unexpected deaths have been reported in SPS, and all described cases have been associated with apnea. Thus, the onset of apnea during SPS should be considered a criterion of high severity and should lead to intensive care unit (ICU) admission for continuous monitoring. In patients with severe disease who are unresponsive to symptomatic treatment with benzodiazepines and baclofen, or in patients with life-threatening complications, early immunotherapy by intravenous immunoglobulins should be considered.
Onset of apneas during SPS should be considered as a signal of possible progression toward acute respiratory failure and sudden death, and should lead to ICU admission for continuous monitoring. Early immunotherapy should be started in such situations, including intravenous immunoglobulins as the first-line treatment.
僵人综合征（SPS）是一种罕见的疾病，其特征为进行性肌肉僵硬、强直及累及轴性肌肉的痉挛。急性呼吸窘迫在这种疾病中鲜有报道。
我们报告一例49岁患有自身免疫性SPS的女性患者，该患者在因反复呼吸暂停继发急性呼吸衰竭发作期间被诊断出来，需要重症监护。
以呼吸暂停发作为表现的急性呼吸衰竭是SPS一种危及生命且不可预测的并发症。其病理生理学尚不清楚。提出的两种机制如下：（1）由于肌肉强直和阵发性肌肉痉挛导致的呼吸暂停，以及（2）阵发性自主神经功能亢进。SPS中曾报道过突然意外死亡，所有描述的病例均与呼吸暂停有关。因此，SPS期间呼吸暂停的发作应被视为高严重性的标准，应导致患者入住重症监护病房（ICU）进行持续监测。对于对苯二氮䓬类药物和巴氯芬对症治疗无反应的重症患者，或有危及生命并发症的患者，应考虑早期通过静脉注射免疫球蛋白进行免疫治疗。
SPS期间呼吸暂停的发作应被视为可能进展为急性呼吸衰竭和猝死的信号，应导致患者入住ICU进行持续监测。在这种情况下应开始早期免疫治疗，包括将静脉注射免疫球蛋白作为一线治疗。
